Chapter 28: The Child with Hematologic or Immunologic Dysfunction

Question 1

1. The regulation of red blood cell (RBC) production is thought to be controlled by which physiologic factor?
   a. Hemoglobin
   b. Tissue hypoxia
   c. Reticulocyte count
   d. Number of RBCs

Answer 1

ANS: B-Tissue hypoxia

Hemoglobin does not directly control RBC production. If there is insufficient hemoglobin to adequately oxygenate the tissue, then erythropoietin may be released. When tissue hypoxia occurs, the kidneys release erythropoietin into the bloodstream. This stimulates the marrow to produce new RBCs. Reticulocytes are immature RBCs. The retic count can be used to monitor hematopoiesis. The number of RBCs does not directly control production. In congenital cardiac disorders with mixed blood flow or decreased pulmonary blood flow, RBC production continues secondary to tissue hypoxia.

Question 2

2. What physiologic defect is responsible for causing anemia?
   a. Increased blood viscosity
   b. Depressed hematopoietic system
   c. Presence of abnormal hemoglobin
   d. Decreased oxygen-carrying capacity of blood

Answer 2

ANS: D-Decreased oxygen-carrying capacity of blood

Anemia is a condition in which the number of red blood cells or hemoglobin concentration is reduced below the normal values for age. This results in a decreased oxygen-carrying capacity of blood. Increased blood viscosity is usually a function of too many cells or of dehydration, not of anemia. A depressed hematopoietic system or abnormal hemoglobin can contribute to anemia, but the definition depends on the decreased oxygen-carrying capacity of the blood.

Question 3

3. A mother states that she brought her child to the clinic because the 3-year-old girl was not keeping up with her siblings. During physical assessment, the nurse notes that the child has pale skin and conjunctiva and has muscle weakness. The hemoglobin on admission is 6.4 g/dl. After notifying the practitioner of the results, what nursing priority intervention should occur next?
   a. Reduce environmental stimulation to prevent seizures.
   b. Have the laboratory repeat the analysis with a new specimen.
   c. Minimize energy expenditure to decrease cardiac workload.
   d. Administer intravenous fluids to correct the dehydration.

Answer 3

ANS: C-Minimize energy expenditure to decrease cardiac workload.

The child has a critically low hemoglobin value. The expected range is 11.5 to 15.5 g/dl. When the oxygen-carrying capacity of the blood decreases slowly, the child is able to compensate by increasing cardiac output. With the increasing workload of the heart, additional stress can lead to cardiac failure. Reduction of environmental stimulation can help minimize energy expenditure, but seizures are not a risk. A repeat hemoglobin analysis is not necessary. The child does not have evidence of dehydration. If intravenous fluids are given, they can further dilute the circulating blood volume and increase the strain on the heart.

Question 4

4. A child with severe anemia requires a unit of red blood cells (RBCs). The nurse explains to the child that the transfusion is necessary for which reason?
   a. Allow her parents to come visit her.
   b. Fight the infection that she now has.
   c. Increase her energy so she will not be so tired.
   d. Help her body stop bleeding by forming a clot (scab).

Answer 4

ANS: C-Increase her energy so she will not be so tired.

The indication for RBC transfusion is risk of cardiac decompensation. When the number of circulating RBCs is increased, tissue hypoxia decreases, cardiac function is improved, and the child will have more energy. Parental visiting is not dependent on transfusion. The decrease in tissue hypoxia will minimize the risk of infection. There is no evidence that the child is currently infected. Forming a clot is the function of platelets.

Question 5

5. An 8-year-old girl is receiving a blood transfusion when the nurse notes that she has developed precordial pain, dyspnea, distended neck veins, slight cyanosis, and a dry cough. These manifestations are most suggestive of what complication?
   a. Air embolism
   b. Allergic reaction
   c. Hemolytic reaction
   d. Circulatory overload

Answer 5

ANS: D-Circulatory overload

The signs of circulatory overload include distended neck veins, hypertension, crackles, a dry cough, cyanosis, and precordial pain. Signs of air embolism are sudden difficulty breathing, sharp pain in the chest, and apprehension. Urticaria, pruritus, flushing, asthmatic wheezing, and laryngeal edema are signs and symptoms of allergic reactions. Hemolytic reactions are characterized by chills, shaking, fever, pain at infusion site, nausea, vomiting, tightness in chest, flank pain, red or black urine, and progressive signs of shock and renal failure.

Question 6

6. What explanation provides the rationale for why iron-deficiency anemia is common during infancy?
   a. Cows milk is a poor source of iron.
   b. Iron cannot be stored during fetal development.
   c. Fetal iron stores are depleted by 1 month of age.
   d. Dietary iron cannot be started until 12 months of age.

Answer 6

ANS: A-Cows milk is a poor source of iron.

Children between the ages of 12 and 36 months are at risk for anemia because cows milk is a major component of their diet, and it is a poor source of iron. Iron is stored during fetal development, but the amount stored depends on maternal iron stores. Fetal iron stores are usually depleted by ages 5 to 6 months. Dietary iron can be introduced by breastfeeding, iron-fortified formula, and cereals during the first 12 months of life.

Question 7

7. What statement best describes iron deficiency anemia in infants?
   a. It is caused by depression of the hematopoietic system.
   b. Diagnosis is easily made because of the infants emaciated appearance.
   c. It results from a decreased intake of milk and the premature addition of solid foods.
   d. Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.

Answer 7

ANS: D-Clinical manifestations are related to a reduction in the amount of oxygen available to tissues.

In iron-deficiency anemia, the childs clinical appearance is a result of the anemia, not the underlying cause. Usually the hematopoietic system is not depressed. The bone marrow produces red blood cells that are smaller and contain less hemoglobin than normal red blood cells. Children who have iron deficiency from drinking excessive quantities of milk are usually pale and overweight. They are receiving sufficient calories but are deficient in essential nutrients. The clinical manifestations result from decreased intake of iron-fortified solid foods and an excessive intake of milk.

Question 8

8. What information should the nurse include when teaching the mother of a 9-month-old infant about administering liquid iron preparations?
   a. Give with meals.
   b. Stop immediately if nausea and vomiting occur.
   c. Adequate dosage will turn the stools a tarry green color.
   d. Allow preparation to mix with saliva and bathe the teeth before swallowing.

Answer 8

ANS: C-Adequate dosage will turn the stools a tarry green color.

The nurse should prepare the mother for the anticipated change in the childs stools. If the iron dose is adequate, the stools will become a tarry green color. A lack of color change may indicate insufficient iron. The iron should be given in two divided doses between meals when the presence of free hydrochloric acid is greatest. Iron is absorbed best in an acidic environment. Vomiting and diarrhea may occur with iron administration. If these occur, the iron should be given with meals, and the dosage reduced and gradually increased as the child develops tolerance. Liquid preparations of iron stain the teeth; they should be administered through a straw and the mouth rinsed after administration.

Question 9

9. Therapeutic management of a 6-year-old child with hereditary spherocytosis (HS) should include which therapeutic intervention?
   a. Perform a splenectomy.
   b. Supplement the diet with calcium.
   c. Institute a maintenance transfusion program.
   d. Increase intake of iron-rich foods such as meat.

Answer 9

ANS: A-Perform a splenectomy.

Splenectomy corrects the hemolysis that occurs in HS. The splenectomy is generally reserved for children older than age 5 years with symptomatic anemia. Supplementation with calcium does not affect the HS. Additional folic acid can prevent deficiency caused by the rapid cell turnover. A maintenance transfusion program suppresses red blood cell formation. At this time, the risks of transfusion are greater than those of a splenectomy. Iron supplementation does not influence the course of HS.

Question 10

10. What condition occurs when the normal adult hemoglobin is partly or completely replaced by abnormal hemoglobin?
    a. Aplastic anemia
    b. Sickle cell anemia
    c. Thalassemia major
    d. Iron deficiency anemia

Answer 10

ANS: B-Sickle cell anemia

Sickle cell anemia is one of a group of diseases collectively called hemoglobinopathies, in which normal adult hemoglobin is replaced by abnormal hemoglobin. Aplastic anemia is a lack of cellular elements being produced. Thalassemia major refers to a variety of inherited disorders characterized by deficiencies in production of certain globulin chains. Iron-deficiency anemia affects red blood cell size and depth of color but does not involve abnormal hemoglobin.

Question 11

11. The parents of a child with sickle cell anemia (SCA) are concerned about subsequent children having the disease. What statement most accurately reflects inheritance of SCA?
    a. SCA is not inherited.
    b. All siblings will have SCA.
    c. Each sibling has a 25% chance of having SCA.
    d. There is a 50% chance of siblings having SCA.

Answer 11

ANS: C-Each sibling has a 25% chance of having SCA.

SCA is inherited as an autosomal recessive disorder. In this inheritance pattern, each child born to these parents has a 25% chance of having the disorder, a 25% chance of having neither SCA nor the trait, and a 50% chance of being heterozygous for SCA (sickle cell trait). SCA is an inherited hemoglobinopathy.

Question 12

12. The clinical manifestations of sickle cell anemia (SCA) are primarily the result of which physiologic alteration?
    a. Decreased blood viscosity
    b. Deficiency in coagulation
    c. Increased red blood cell (RBC) destruction
    d. Greater affinity for oxygen

Answer 12

ANS: C-Increased red blood cell (RBC) destruction

The clinical features of SCA are primarily the result of increased RBC destruction and obstruction caused by the sickle-shaped RBCs. When the sickle cells change shape, they increase the viscosity in the area where they are involved in the microcirculation. SCA does not have a coagulation deficit. Sickled red cells have decreased oxygen-carrying capacity and transform into the sickle shape in conditions of low oxygen tension.

Question 13

13. A school-age child is admitted in vasoocclusive sickle cell crisis (pain episode). The childs care should include which therapeutic interventions?
    a. Hydration and pain management
    b. Oxygenation and factor VIII replacement
    c. Electrolyte replacement and administration of heparin
    d. Correction of alkalosis and reduction of energy expenditure

Answer 13

ANS: A-Hydration and pain management

The management of crises includes adequate hydration, pain management, minimization of energy expenditures, electrolyte replacement, and blood component therapy if indicated. Factor VIII is not indicated in the treatment of vasoocclusive sickle cell crisis. Oxygen may prevent further sickling, but it is not effective in reversing sickling because it cannot reach the clogged blood vessels. Also, prolonged oxygen can reduce bone marrow activity. Heparin is not indicated in the treatment of vasoocclusive sickle cell crisis. Electrolyte replacement should accompany hydration. The acidosis will be corrected as the crisis is treated. Energy expenditure should be minimized to improve oxygen utilization. Acidosis, not alkalosis, results from hypoxia, which also promotes sickling.

Question 14

14. A child with sickle cell anemia (SCA) develops severe chest and back pain, fever, a cough, and dyspnea. What should be the first action by the nurse?
    a. Administer 100% oxygen to relieve hypoxia.
    b. Notify the practitioner because chest syndrome is suspected.
    c. Infuse intravenous antibiotics as soon as cultures are obtained.
    d. Give ordered pain medication to relieve symptoms of pain episode.

Answer 14

ANS: B-Notify the practitioner because chest syndrome is suspected.

These are the symptoms of chest syndrome, which is a medical emergency. Notifying the practitioner is the priority action. Oxygen may be indicated; however, it does not reverse the sickling that has occurred. Antibiotics are not indicated initially. Pain medications may be required, but evaluation by the practitioner is the priority.

Question 15

15. In a child with sickle cell anemia (SCA), adequate hydration is essential to minimize sickling and delay the vasoocclusion and hypoxiaischemia cycle. What information should the nurse share with parents in a teaching plan?
    a. Encourage drinking.
    b. Keep accurate records of output.
    c. Check for moist mucous membranes.
    d. Monitor the concentration of the childs urine.

Answer 15

ANS: C-Check for moist mucous membranes.

Children with SCA have impaired kidney function and cannot concentrate urine. Parents are taught signs of dehydration and ways to minimize loss of fluid to the environment. Encouraging drinking is not specific enough for parents. The nurse should give the parents and child a target fluid amount for each 24-hour period. Accurate monitoring of output may not reflect the childs fluid needs. Without the ability to concentrate urine, the child needs additional intake to compensate. Dilute urine and specific gravity are not valid signs of hydration status in children with SCA.

Question 16

16. What statement best describes b-thalassemia major (Cooley anemia)?
    a. It is an acquired hemolytic anemia.
    b. Inadequate numbers of red blood cells (RBCs) are present.
    c. Increased incidence occurs in families of Mediterranean extraction.
    d. It commonly occurs in individuals from West Africa.

Answer 16

ANS: C-Increased incidence occurs in families of Mediterranean extraction.

Individuals who live near the Mediterranean Sea and their descendants have the highest incidence of thalassemia. Thalassemia is inherited as an autosomal recessive disorder. An overproduction of RBCs occurs. Although numerous, the red blood cells are relatively unstable. Sickle cell disease is common in blacks of West African descent.

Question 17

17. What therapeutic intervention is most appropriate for a child with b-thalassemia major?
    a. Oxygen therapy
    b. Supplemental iron
    c. Adequate hydration
    d. Frequent blood transfusions

Answer 17

ANS: D-Frequent blood transfusions

The goal of medical management is to maintain sufficient hemoglobin (>9.5 g/dl) to prevent bone marrow expansion. This is achieved through a long-term transfusion program. Oxygen therapy and adequate hydration are not beneficial in the overall management of thalassemia. The child does not require supplemental iron. Iron overload is a problem because of frequent blood transfusions, decreased production of hemoglobin, and increased absorption from the gastrointestinal tract.

Question 18

18. Iron overload is a side effect of chronic transfusion therapy. What treatment assists in minimizing this complication?
    a. Magnetic therapy
    b. Infusion of deferoxamine
    c. Hemoglobin electrophoresis
    d. Washing red blood cells (RBCs) to reduce iron

Answer 18

ANS: B-Infusion of deferoxamine

Deferoxamine infusions in combination with vitamin C allow the iron to remain in a more chelatable form. The iron can then be excreted more easily. Use of magnets does not remove additional iron from the body. Hemoglobin electrophoresis is used to confirm the diagnosis of hemoglobinopathies; it does not affect iron overload. Washed RBCs remove white blood cells and other proteins from the unit of blood; they do not affect the iron concentration.

Question 19

19. In which condition are all the formed elements of the blood simultaneously depressed?
    a. Aplastic anemia
    b. Sickle cell anemia
    c. Thalassemia major
    d. Iron deficiency anemia

Answer 19

ANS: A-Aplastic anemia

Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Sickle cell anemia is a hemoglobinopathy in which normal adult hemoglobin is partly or completely replaced by abnormal sickled hemoglobin. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin chains. Iron-deficiency anemia results in a decreased amount of circulating red cells.

Question 20

20. For children who do not have a matched sibling bone marrow donor, the therapeutic management of aplastic anemia includes what intervention?
    a. Antibiotics
    b. Antiretroviral drugs
    c. Iron supplementation
    d. Immunosuppressive therapy

Answer 20

ANS: D-Immunosuppressive therapy

It is thought that aplastic anemia may be an autoimmune disease. Immunosuppressive therapy, including antilymphocyte globulin, antithymocyte globulin, cyclosporine, granulocyte colony-stimulating factor, and methylprednisone, has greatly improved the prognosis for patients with aplastic anemia. Antibiotics are not indicated as the management. They may be indicated for infections. Antiretroviral drugs and iron supplementation are not part of the therapy.

Question 21

21. What statement is descriptive of most cases of hemophilia?
    a. X-linked recessive deficiency of platelets causing prolonged bleeding
    b. X-linked recessive inherited disorder in which a blood clotting factor is deficient
    c. Autosomal dominant deficiency of a factor involved in the blood-clotting reaction
    d. Y-linked recessive inherited disorder in which the red blood cells become moon shaped

Answer 21

ANS: B-X-linked recessive inherited disorder in which a blood clotting factor is deficient

The inheritance pattern in 80% of all the cases of hemophilia is X-linked recessive. The two most common forms of the disorder are factor VIII deficiency (hemophilia A, or classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas disease). The disorder involves coagulation factors, not platelets. The disorder does not involve red blood cells or the Y chromosome.

Question 22

22. The nurse is teaching the family of a child, age 8 years, with moderate hemophilia about home care. What should the nurse tell the family to do to minimize joint injury?
    a. Administer nonsteroidal anti-inflammatory drugs (NSAIDs).
    b. Administer DDAVP (synthetic vasopressin).
    c. Provide intravenous (IV) infusion of factor VIII concentrates.
    d. Encourage elevation and application of ice to the involved joint.

Answer 22

ANS: C-Provide intravenous (IV) infusion of factor VIII concentrates.

Parents are taught home infusion of factor VIII concentrate. For moderate and severe hemophilia, prompt IV administration is essential to prevent joint injury. NSAIDs are effective for pain relief. They must be given with caution because they inhibit platelet aggregation. A factor VIII level of 30% is necessary to stop bleeding. DDAVP can raise the factor VIII level fourfold. Moderate hemophilia is defined by a factor VIII activity of 4.9. A fourfold increase would not meet the 30% level. Ice and elevation are important adjunctive therapy, but factor VIII is necessary.

Question 23

23. What condition is an acquired hemorrhagic disorder that is characterized by excessive destruction of platelets?
    a. Aplastic anemia
    b. Thalassemia major
    c. Idiopathic thrombocytopenic purpura
    d. Disseminated intravascular coagulation

Answer 23

ANS: C-Idiopathic thrombocytopenic purpura

Idiopathic thrombocytopenic purpura is an acquired hemorrhagic disorder characterized by an excessive destruction of platelets, discolorations caused by petechiae beneath the skin, and normal bone marrow. Aplastic anemia refers to a bone marrow failure condition in which the formed elements of the blood are simultaneously depressed. Thalassemia major is a group of blood disorders characterized by deficiency in the production rate of specific hemoglobin chains. Disseminated intravascular coagulation is characterized by diffuse fibrin deposition in the microvasculature, consumption of coagulation factors, and endogenous generation of thrombin and plasma.

Question 24

24. Care for the child with acute idiopathic thrombocytopenic purpura (ITP) includes which therapeutic intervention?
    a. Splenectomy
    b. Intravenous administration of anti-D antibody
    c. Use of nonsteroidal anti-inflammatory drugs (NSAIDs)
    d. Helping child participate in sports

Answer 24

ANS: B-Intravenous administration of anti-D antibody

Anti-D antibody causes an increase in platelet count approximately 48 hours after administration. Splenectomy is reserved for chronic severe ITP not responsive to pharmacologic management. NSAIDs are not used in ITP. Both NSAIDs and aspirin interfere with platelet aggregation. The nurse works with the child and parents to choose quiet activities while the platelet count is below 100,000/mm3.

Question 25

25. A toddler is diagnosed with chronic benign neutropenia. The parents are being taught about caring for their child. What information is important to include? a. Avoid large indoor crowds and people who are ill. b. Parenteral antibiotics are necessary to control disease. c. Frequent rest periods are needed during the daytime. d. List the side effects of corticosteroids used to decrease inflammation.

Answer 25

ANS: A- Avoid large indoor crowds and people who are ill. The parents are taught to minimize risk of infection by avoiding crowded areas and individuals who are ill. Parents are also cautioned about when to notify their practitioner and administration of granulocyte colony-stimulating factor, if indicated. Antibiotics are not needed unless the child has an infection. The toddler does not need any additional rest as a result of the neutropenia. Corticosteroids are not indicated.

Question 26

26. The majority of children in the United States with human immunodeficiency virus (HIV) infection acquired the disease by which means? a. Through sexual contact b. From a blood transfusion c. By using intravenous (IV) drugs d. Perinatally from their mothers

Answer 26

ANS: D-Perinatally from their mothers More than 90% of the children with HIV under 13 years who were reported to the Centers for Disease Control and Prevention acquired the infection during the perinatal period. With intervention, the number of children infected can be decreased. Sexual contact and IV drug use are the leading causes of infection in the 14- to 19-year age group. This number is less than the number of cases in the under 13-year age group. Transfusion has accounted for 3% to 6% of all pediatric acquired immunodeficiency syndrome cases to date. Before 1985 and routine screening of donated blood products, children with hemophilia were at great risk from pooled plasma products.

Question 27

27. A young child with human immunodeficiency virus (HIV) is receiving several antiretroviral drugs. What is the purpose of these drugs? a. Cure the disease. b. Delay disease progression. c. Prevent spread of infection. d. Treat Pneumocystis carinii pneumonia.

Answer 27

ANS: B-Delay disease progression. Although not a cure, these antiretroviral drugs can suppress viral replication, preventing further deterioration of the immune system, and delay disease progression. At this time, cure is not possible. Antiretroviral drugs do not prevent the spread of the disease. P. carinii prophylaxis is accomplished with antibiotics.

Question 28

28. The nurse is planning care for an adolescent with acquired immunodeficiency syndrome. What is the priority nursing goal? a. Prevent infection. b. Prevent secondary cancers. c. Identify source of infection. d. Restore immunologic defenses.

Answer 28

ANS: A-Prevent infection. As a result of the immunocompromise that is associated with human immunodeficiency virus (HIV) infection, the prevention of infection is paramount. Although certain precautions are justified in limiting exposure to infection, these must be balanced with the concern for the childs normal developmental needs. Preventing secondary cancers is not currently possible. Case finding is not a priority nursing goal in planning care for an individual. Current drug therapy is affecting the disease progression; although not a cure, these drugs can suppress viral replication, preventing further deterioration but not actually restoring immunologic defenses.

Question 29

29. The school nurse is informed that a child with human immunodeficiency virus (HIV) infection will be attending school soon. What is an important nursing intervention to include in the plan of care? a. Carefully follow universal precautions. b. Inform the parents of the other children. c. Determine how the child became infected. d. Reassure other children that they will not become infected.

Answer 29

ANS: A-Carefully follow universal precautions. Universal precautions are necessary to prevent further transmission of the disease. Informing the parents of the other children would violate the childs right to privacy. It is not within the role of the school nurse to determine how the child became infected. Reassuring other children that they will not become infected violates the childs privacy. General health classes can discuss prevention of HIV transmission.

Question 30

30. What condition is an inherited immunodeficiency disorder characterized by absence of both humoral and cell-mediated immunity? a. Fanconi syndrome b. Wiskott-Aldrich syndrome c. Acquired immunodeficiency syndrome (AIDS) d. Severe combined immunodeficiency syndrome (SCIDS)

Answer 30

ANS: D-Severe combined immunodeficiency syndrome (SCIDS) SCIDS is a genetic disorder that results in deficits of both humoral and cellular immunity. Fanconi syndrome is a hereditary disorder of red blood cell production. Wiskott-Aldrich syndrome is an X-linked recessive disorder with selected deficiencies of T and B lymphocytes. AIDS is not inherited.

Question 31

31. The nurse is preparing a community outreach program about the prevention of iron-deficiency anemia in infants. What statement should the nurse include in the program? a. Whole milk can be introduced into the infants diet in small amounts at 6 months. b. Iron supplements cannot be given until the infant is older than 1 year of age. c. Iron-fortified cereal should be introduced to the infant at 2 months of age. d. Breast milk or iron-fortified formula should be used for the first 12 months.

Answer 31

ANS: D-Breast milk or iron-fortified formula should be used for the first 12 months. Prevention, the primary goal in iron-deficiency anemia, is achieved through optimal nutrition and appropriate iron supplements. The American Academy of Pediatrics recommends feeding an infant only breast milk or iron-fortified formula for the first 12 months of life. Whole cows milk should not be introduced until after 12 months, iron supplements can be given during the first year of life, and iron-fortified cereals should not be introduced until the infant is 4 to 6 months old.

Question 32

32. A 5-year-old child is admitted to the hospital in a sickle cell crisis. The child has been alert and oriented but in severe pain. The nurse notes that the child is complaining of a headache and is having unilateral hemiplegia. What action should the nurse implement? a. Notify the health care provider. b. Place the child on bed rest. c. Administer a dose of hydrocodone (Vicodin). d. Start O2 per the hospitals protocol.

Answer 32

ANS: A-Notify the health care provider. Any number of neurologic symptoms can indicate a minor cerebral insult, such as headache, aphasia, weakness, convulsions, visual disturbances, or unilateral hemiplegia. Loss of vision is usually the result of progressive retinopathy and retinal detachment. The nurse should notify the health care provider.

Question 33

33. What pain medication is contraindicated in children with sickle cell disease (SCD)? a. Meperidine (Demerol) b. Hydrocodone (Vicodin) c. Morphine sulfate d. Ketorolac (Toradol)

Answer 33

ANS: A-Meperidine (Demerol) Meperidine (pethidine [Demerol]) is not recommended. Normeperidine, a metabolite of meperidine, is a central nervous system stimulant that produces anxiety, tremors, myoclonus, and generalized seizures when it accumulates with repetitive dosing. Patients with SCD are particularly at risk for normeperidine-induced seizures.

Question 34

34. In anticipation of the admission of a child with hereditary spherocytosis (HS) who is experiencing an aplastic crisis, what action should the nurse plan? a. Secure an isolation room. b. Prepare for a transfusion of packed red blood cells. c. Anticipate preoperative preparation for a splenectomy. d. Gather equipment and medication for treatment of shock.

Answer 34

ANS: B-Prepare for a transfusion of packed red blood cells. In hereditary spherocytosis, aplastic crisis results in a sudden cessation of RBC production by the bone marrow. Hemoglobin and hematocrit values drop rapidly, which results in severe anemia. Transfusion support may be needed, and close monitoring of the childs cardiovascular status is necessary. The nurse should prepare for a transfusion of packed red blood cells initially. An isolation room is not needed, splenectomy would not be done at this time, and the child will not be in shock.

Question 35

35. A child with hemophilia A will have which abnormal laboratory result? a. PT (ProTime) b. Platelet count c. Fibrinogen level d. PTT (partial thromboplastin time)

Answer 35

ANS: D- PTT (partial thromboplastin time) The basic defect of hemophilia A is a deficiency of factor VIII. The partial thromboplastin time measures abnormalities in the intrinsic pathway (abnormalities in factors I, II, V, VIII, IX, X, XII, HMK, and KAL). The prothrombin time measures abnormalities of the extrinsic pathway (abnormalities in factors I, II, V, VII, and X). Fibrinogen level is not dependent on the intrinsic pathway. Platelets are not affected with hemophilia A.

Question 36

36. A child with hemophilia A is scheduled for surgery. What precautions should the nurse institute with this child? a. Handle the child gently when transferring to a cart. b. Caution the child not to brush his teeth before surgery. c. Use tape sparingly on postoperative dressings. d. Do not administer analgesics before surgery.

Answer 36

ANS: A-Handle the child gently when transferring to a cart. The goal of prevention of bleeding episodes is directed toward decreasing the risk of injury. The child should be handled carefully when transferring to a cart. Brushing teeth, use of tape, and giving analgesics will not risk a bleeding episode.

Question 37

37. Nursing strategies to improve the growth and development of the child with human immunodeficiency virus (HIV) infection should include what? a. Provide only those foods that the child feels like eating. b. Fortify foods with nutritional supplements to maximize quality of intake. c. Weigh the child and measure height and muscle mass on a daily basis. d. Provide high-fat and high-calorie meals and snacks to meet body requirements for growth.

Answer 37

ANS: B- Fortify foods with nutritional supplements to maximize quality of intake. HIV infection often leads to marked failure to thrive and multiple nutritional deficiencies. Nutritional management may be difficult because of recurrent illness, diarrhea, and other physical problems. The nurse should implement intensive nutritional interventions if the childs growth begins to slow or weight begins to decrease. Fortifying foods with nutritional supplements will maximize quality of intake. The child does not need to be weighed daily, and high-fat meals and snacks should not be encouraged.

Question 38

38. What medication is classified as an antiretroviral? a. Dapsone (Aczone) b. Pentamidine (Pentam) c. Didanosine (Videx) d. Trimethoprimsulfamethoxazole (Bactrim)

Answer 38

ANS: C-Didanosine (Videx) Classes of antiretroviral agents include nucleoside reverse transcriptase inhibitors (e.g., zidovudine, didanosine, stavudine, lamivudine, abacavir), nonnucleoside reverse transcriptase inhibitors (e.g., nevirapine, delavirdine, efavirenz), and protease inhibitors (e.g., indinavir, saquinavir, ritonavir, nelfinavir, amprenavir, lopinavir, ritonavir). Dapsone, pentamidine, and Bactrim are anti-infectives.

Question 39

39. The nurse is caring for a child with hemophilia A. The childs activity is as tolerated. What activity is contraindicated for this child? a. Ambulating to the cafeteria b. Active range of motion c. Ambulating to the playroom d. Passive range of motion exercises

Answer 39

ANS: D- Passive range of motion exercises Passive range of motion exercises should never be part of an exercise regimen after an acute episode because the joint capsule could easily be stretched and bleeding could recur. Active range of motion exercises are best so that the patient can gauge his or her own pain tolerance. The child can ambulate to the playroom or the cafeteria.

Question 40

40. What condition precipitates polycythemia? a. Dehydration b. Severe infections c. Immunosuppression d. Prolonged tissue hypoxia

Answer 40

ANS: D-Prolonged tissue hypoxia Oxygen transport depends on both the number of circulating RBCs and the amount of normal hemoglobin in the cell. This explains why polycythemia (increase in the number of erythrocytes) occurs in conditions characterized by prolonged tissue hypoxia, such as cyanotic heart defects. Dehydration, severe infections, or immunosuppression will not precipitate polycythemia.

Question 41

41. The clinic nurse is evaluating lab results for a child. What recorded hemoglobin (Hgb) result is considered within the normal range? a. 9 g/dl b. 10 g/dl c. 11 g/dl d. 12 g/dl

Answer 41

ANS: D-12 g/dl Normal hemoglobin (Hgb) determination is 11.5 to 15.5 g/dl.

Question 42

42. The clinic nurse is evaluating lab results for a child. What recorded hematocrit (Hct) result is considered within the normal range? a. 30% b. 40% c. 50% d. 60%

Answer 42

ANS: B-40% Normal hematocrit (Hct) is 35% to 45%.

Question 43

43. The nurse is caring for a school-age child with severe anemia and activity intolerance. What diversional activity should the nurse plan for this child? a. Playing a musical instrument b. Playing board or card games c. Participating in a game of table tennis d. Participating in decorating the hospital room

Answer 43

ANS: B- Playing board or card games Plan diversional activities that promote rest but prevent boredom and withdrawal. Because short attention span, irritability, and restlessness are common in anemia and increase stress demands on the body, plan appropriate activities such as playing board or card games. Playing a musical instrument, participating in a game of table tennis, or decorating the hospital room would cause undue exertion.

Question 44

44. The nurse is preparing to administer a unit of packed red blood cells to a hospitalized child. What is an appropriate action that applies to administering blood? a. Take the vital signs every 15 minutes while blood is infusing. b. Use blood within 1 hour of its arrival from the blood bank. c. Administer the blood with 5% glucose in a piggyback setup. d Administer the first 50 ml of blood slowly and stay with the child.

Answer 44

ANS: D-Administer the first 50 ml of blood slowly and stay with the child. The nurse should administer the first 50 ml of blood or initial 20% of volume (whichever is smaller) slowly and stay with the child. Vitals signs should be taken 15 minutes after initiation and then every hour, not every 15 minutes. Blood should be used within 30 minutes, not 1 hour. Normal saline, not 5% glucose, should be the IV solution.

Question 45

45. What rationale explains why prolonged use of oxygen should be discouraged in a child with anemia? a. Prolonged use of oxygen can decrease erythropoiesis. b. Prolonged use of oxygen can interfere with iron production. c. Prolonged use of oxygen interferes with a childs appetite. d. Prolonged use of oxygen can affect the synthesis of hemoglobin.

Answer 45

ANS: A-Prolonged use of oxygen can decrease erythropoiesis. Oxygen administration is of limited value, because each gram of hemoglobin is able to carry a limited amount of the gas. In addition, prolonged use of supplemental oxygen can decrease erythropoiesis. Prolonged use of oxygen does not interfere with iron production, a childs appetite, or affect the synthesis of hemoglobin.

Question 46

46. The nurse is teaching a parent of an infant to limit the amount of formula to encourage the intake of iron-rich food. What amount should the nurse teach to the parent? a. 500 ml b. 750 ml c. 1000 ml d. 1250 ml

Answer 46

ANS: C-1000 ml The nurse should teach the parent to limit the amount of formula to no more than 1 1/day to encourage intake of iron-rich solid foods.

Question 47

47. A child with sickle cell disease is in a vasoocclusive crisis. What nonpharmacologic pain intervention should the nurse plan? a. Exercise as a distraction b. Heat to the affected area c. Elevation of the extremity d. Cold compresses to the affected area

Answer 47

ANS: B-Heat to the affected area Frequently, heat to the affected area is soothing. Cold compresses are not applied to the area because doing so enhances vasoconstriction and occlusion. Bed rest is usually well tolerated during a crisis, although the actual rest obtained depends a great deal on pain alleviation and the use of organized schedules of nursing care. Although the objective of bed rest is to minimize oxygen consumption, some activity, particularly passive range of motion exercises, is beneficial to promote circulation. Usually the best course is to let children determine their activity tolerance. Elevating the extremity will not help in sickle cell disease.

Question 48

48. What immunoglobulin pattern does the nurse expect in a child recently diagnosed with Wiskott-Aldrich syndrome? a. Diminished levels of IgG b. Diminished levels of IgA c. Diminished levels of IgM d. Diminished levels of IgE

Answer 48

ANS: C-Diminished levels of IgM The level of IgM is diminished early in the course of the disease, but levels of IgG, IgA, and IgE may be elevated initially.